Treatment of Supratentorial Primitive Neuroectodermal Tumors (PNETs) in Children

OBJECTIVE: Strategies for managing supratentorial primitive neuroectodermal tumors(PNETs) in children include surgical resection, craniospinal irradiation and chemotherapy. This study is performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. METHODS: We have reviewed all medical records and pathology slides of six children(four males and two females) with supratentorial PNET from November, 1987 to May, 2003. The extent of resection was confirmed by computed tomography and magnetic resonance studies. RESULTS: The patients were aged 1 to 13 years and treated postoperatively with/without adjuvant therapy. Tumor location included was four cortical, one gangliobasal, and one pineal region. The presenting symptoms and signs consisted of increased intracranial pressure and focal neurological deficits such as seizure and hemiparesis. The treatment consisted of surgical resection alone in one patient, postoperative radiotherapy in one patient, postoperative chemotherapy in one, and postoperative radiotherapy with chemotherapy in three. Five patients lived more than 12 months after diagnosis and one patient among them has been living more than 5 years after diagnosis. CONCLUSION: We can improve the survival and prognosis of supratentorial PNET patients by radical gross total resection of tumor followed by craniospinal irradiation and aggressive chemotherapy. First of all, gross total resection of tumor is the most important among many factors.

Ictal SPECT-guided Epilepsy Surgery in a Patient with Forme Fruste Tuberous Sclerosis

Tuberous sclerosis is an autosomal dominant disease characterised by hamartomas (tubers) in many organ systems and the four major intracranial manifestations including cortical tubers, white matter abnormalities, subependymal nodules and subependymal giant cell astrocytoma. But there is immense variability in the clinical presentation of tuberous sclerosis and many incomplete forms (formes frustes) exist. Almost all patients with tuberous sclerosis have seizures and mental retardation. The authors experienced a 7-year-old boy with medically intractable epilepsy without any skin lesion or mental retardation. In terms of surgical standpoint for determination of extent of resection, corticectomy on the overriding cortex of right premotor and lesionectomy of periventricular calcified lesion were performed according to ictal single photon emission computed tomography(SPECT), which showed hyperperfusion in the subcortical and calcified area. Histopathologic findings showed a few cytologically abnormal neurons with extensive gliosis, containing many Rosenthal fibers, reactive astrocytes and dense calcification, composing of abundant calcospherites which suggested forme fruste tuberous sclerosis. During the follow-up period of eighteen months, seizure was free after surgery.